International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency

J Allergy Clin Immunol. 2012 Feb;129(2):308-20. doi: 10.1016/j.jaci.2011.11.025. Epub 2011 Dec 24.

Abstract

Background: There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH).

Objective: We sought to elaborate guidelines for optimizing the management of gynecologic/obstetric events in female patients with HAE-C1-INH.

Methods: A roundtable discussion took place at the 6th C1 Inhibitor Deficiency Workshop (May 2009, Budapest, Hungary). A review of related literature in English was performed.

Results: Contraception: Estrogens should be avoided. Barrier methods, intrauterine devices, and progestins can be used. Pregnancy: Attenuated androgens are contraindicated and should be discontinued before attempting conception. Plasma-derived human C1 inhibitor concentrate (pdhC1INH) is preferred for acute treatment, short-term prophylaxis, or long-term prophylaxis. Tranexamic acid or virally inactivated fresh frozen plasma can be used for long-term prophylaxis if human plasma-derived C1-INH is not available. No safety data are available on icatibant, ecallantide, or recombinant human C1-INH (rhC1INH). Parturition: Complications during vaginal delivery are rare. Prophylaxis before labor and delivery might not be clinically indicated, but pdhC1INH therapeutic doses (20 U/kg) should be available. Nevertheless, each case should be treated based on HAE-C1-INH symptoms during pregnancy and previous labors. pdhC1INH prophylaxis is advised before forceps or vacuum extraction or cesarean section. Regional anesthesia is preferred to endotracheal intubation. Breast cancer: Attenuated androgens should be avoided. Antiestrogens can worsen angioedema symptoms. In these cases anastrozole might be an alternative. Other issues addressed include special features of HAE-C1-INH treatment in female patients, genetic counseling, infertility, abortion, lactation, menopause treatment, and endometrial cancer.

Conclusions: A consensus for the management of female patients with HAE-C1-INH is presented.

Publication types

  • Consensus Development Conference
  • Practice Guideline
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Breast Neoplasms / complications
  • Chemoprevention
  • Complement C1 Inactivator Proteins / deficiency*
  • Complement C1 Inhibitor Protein
  • Contraception
  • Delivery, Obstetric
  • Female
  • Genetic Counseling
  • Genital Diseases, Female / complications
  • Hereditary Angioedema Types I and II* / complications
  • Hereditary Angioedema Types I and II* / diagnosis
  • Hereditary Angioedema Types I and II* / drug therapy
  • Hereditary Angioedema Types I and II* / genetics
  • Humans
  • Infant
  • Infant, Newborn
  • Lactation
  • Menopause
  • Menstruation
  • Pregnancy
  • Pregnancy Complications, Cardiovascular* / diagnosis
  • Pregnancy Complications, Cardiovascular* / drug therapy
  • Prenatal Diagnosis

Substances

  • Complement C1 Inactivator Proteins
  • Complement C1 Inhibitor Protein
  • SERPING1 protein, human